Mouthpiece Ventilation in Patients with Neuromuscular Disease: A Brief Clinical Review

Review Article

Phys Med Rehabil Int. 2014;1(3): 4.

Mouthpiece Ventilation in Patients with Neuromuscular Disease: A Brief Clinical Review

Nicolini A, Russo D , Barlascini CO*, Sclifò F§, Grecchi B^, Garuti GC°, Banfi PI°°

Respiratory Rehabilitation Unit and ALS Centre, Italy

*Public Health Department, Italy

§ Allergy & Respiratory Diseases Clinic, University of Genoa, Italy

^Physical Medicine and Rehabilitation, Italy

°Pulmonary Department Santa Maria Bianca, Italy

°°Don Gnocchi Foundation IRCSS, Italy

*Corresponding author: Nicolini Antonello, Respiratory Rehabilitation Unit and ALS Centre, Italy

Received: July 31, 2014; Accepted: September 22, 2014; Published: September 25, 2014


The Open-circuit Mouthpiece Ventilation (MPV) is a mode of ventilation that uses a mouthpiece interface which the patient holds with the lips when he wants to be supported during inspiration. There exists a poor understanding of this method's benefits compared to other modalities. Non-invasive ventilation (NIV) is sometimes reported as suboptimal in neuromuscular disease patients due to excessive secretions in the airways , hypercapnia due to inadequate ventilator settings , or because of a lack of tolerance of the interface. Interfaces that cover the nose and / or mouth and nose are the most commonly used, but may cause skin lesions and claustrophobia. Many of these drawbacks can be avoided by using a mouthpiece to administer the NIV. The MPV is used by many patients as daytime ventilatory support in combination with other modes of ventilation interface effective for night ventilation. There are two models mouthpiece of different sizes 15 and 22 mm .NIV has been used for years in patients with neuromuscular disorders as a viable alternative to continuous ventilatory support via tracheotomy tube. NIV is associated with a reduced risk of pneumonia and other respiratory complications. Its use in the volumetric mode allows air-stacking to improve cough. The mouthpiece interface facilitates speech and swallowing, factors that lead to a better quality of life for patients. This review aims to highlight the indications, along with the advantages and disadvantages of MPV.

Keywords: Non-invasive ventilation; Mouthpiece; Open-circuit mouthpiece ventilation (MPV); Neuromuscular disease; Ventilator settings


Neuromuscular diseases represent a heterogeneous group of disorders of the muscle, nerve, and/or some neuromuscular junction. The respiratory muscles are rarely spared in neuromuscular diseases even if the type of muscle involvement, severity, and time course greatly varies among the different diseases [1]. The most common neuromuscular diseases in childhood are Duchenne muscular dystrophy (DMD), spinal muscular atrophy (SMA ) and congenital myophathy, congenital muscular dystrophy ( CMD ) . In adults, amyotrophic lateral sclerosis, myotonic myopathy (Steiner's disease) and limb-girdle muscular dystrophy (LGMD) are the most common neuromuscular diseases which can benefit from NIV treatment according their progressiveness are reported in the Table 1[2].Before 1953, the non-invasive ventilation (NIV) was practiced through the use of negative pressure ventilators : "iron lung ","armor". Despite of their great success as a continuous ventilator support, ventilation through tracheotomy became the standard since the epidemic Danish polio in 1952, because it was possible to move patients and secretions could be easily managed [3]. In 1953 patients who used lungs steel or armor began to use the ventilation mouthpiece during the day. In the United States intermittent positive pressure ventilation (IPPV) through a mouthpiece has been the accepted practice for patients requiring continuous ventilatory support as an alternative to mechanical ventilation via a tracheotomy tube [3]. The portable ventilator manufactured by Bantam Harris Thompson in 1956 was another turning point. Mouthpiece use slowly grew in patients with severe ventilatory defect due to restrictive illnesses (vital capacity lower than 500 ml) and with inefficient cough [3]. The result was a reduced dependence on the ventilator with less hyper secretion [3, 4]. Non invasive ventilation via mouthpiece was used in 257 patients requiring continuous ventilatory support at the Goldwater Memorial Hospital from 1968 to 1987 with excellent results. [5]. Despite remarkable results by Dr. JR. Bach's group [ 3,4,5 ], few centers in the United States used the ventilation via mouthpiece in neuromuscular patients .Until ten years ago there were only sporadic reports of NIV mouthpiece in this patient group whose numbers have continued to increase with time. Server a et al [6] and Toussaint et al. [7, 8] changed this.